iatroX Rounds #29
The clues
- 1
A 32-year-old man presents with persistent fatigue and an irritating, generalised itchy sensation that is worse at night.
- 2
He has a ten-year history of bloody diarrhoea and abdominal urgency, currently managed with maintenance 5-aminosalicylates.
- 3
Initial blood tests show a serum bilirubin of 22 umol/L, alkaline phosphatase of 450 U/L, and an ALT of 55 U/L.
- 4
On examination, there is no jaundice or hepatomegaly, but numerous excoriations are visible on his limbs from scratching.
- 5
Screening for autoimmune liver disease shows negative anti-mitochondrial antibodies, but p-ANCA is detected in the serum.
- 6
Magnetic resonance cholangiopancreatography shows a series of multifocal, short-segment strictures alternating with normal or slightly dilated segments.
Primary sclerosing cholangitis is a chronic liver disease characterised by inflammation and progressive fibrosis of the bile ducts. It leads to cholestasis and eventually liver failure, and is highly associated with ulcerative colitis.
- Cholestatic liver function tests with predominant alkaline phosphatase rise
- Pruritus and fatigue as common presenting symptoms
- Strong association with inflammatory bowel disease, particularly ulcerative colitis
- Negative anti-mitochondrial antibody distinguishes it from primary biliary cholangitis
for education and entertainment. not medical advice.